Anti-UFSP2 Antibody

The UFSP2 protein (Ufm1-specific protease 2) is a critical regulator of 'ufmylation, ' a post-translational modification where the ubiquitin-like modifier Ufm1 is attached to target proteins. Identified in the mid-2000s, UFSP2 serves as the primary de-UFMylase, the enzyme responsible for removing Ufm1 from substrates, while working alongside UFSP1 to facilitate Ufm1 maturation. Its clinical relevance is significant: mutations in the UFSP2 gene are the established cause of Beukes familial hip dysplasia (an autosomal dominant skeletal disorder) and have been increasingly linked to recessive forms of early-onset epileptic encephalopathy and developmental delay. By regulating the ufmylation pathway, UFSP2 helps cells manage endoplasmic reticulum (ER) stress and maintain protein homeostasis, making it an important subject in research for treating genetic, neurodevelopmental, and skeletal pathologies.

Store at 4°C in the dark. Under these conditions, the antibodies should have a shelf life of at least twelve months, provided they remain sterile. For longer term storage, aliquot and freeze to avoid freeze-thaw of the antibody.

Each new lot of this antibody is tested to confirm that it recognizes a single immunoreactive band of expected molecular weight when used in Western blot.